Search Database ICD-O-3 Code Lists

Name

Acute myeloid leukemia with mutated RUNX1 (see 9861/3 prior to 2021)

ICD-O-3 Morphology

9879/3: Acute myeloid leukemia with mutated RUNX1
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C421
Primary site must be bone marrow (C421)

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2021 and later. For cases diagnosed prior to 2021 see code: 9861/3.)

Acute myeloid leukemia with mutated RUNX1 is part of the Acute myeloid leukemia (AML) lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B6)

Patients usually present with lower hemoglobin and lactate dehydrogenase levels and lower white blood cell and peripheral blood blast cell counts.

Cases with a history of prior therapy (in particular radiation therapy), MDS, or a myelodysplastic/myeloproliferative neoplasm may harbor RUNX1 mutations, but are excluded from this category.

If this leukemia and myeloid sarcoma (9930/3) occur during the same clinical workup, this is one primary, the leukemia.

If the myeloid sarcoma occurs after the diagnosis of the leukemia, that is a manifestation of the leukemia and is the same primary.

See Multiple Primary Rule M3

Diagnostic Confirmation

This AML is part of the "AML with recurrent genetic abnormalities" group. Since this AML is diagnosed based on genetics, diagnostic confirmation will always be 3.

Module Rule

None

Alternate Names

None

Definition

Acute myeloid leukemia (AML) with mutated RUNX1 is a de novo leukemia with greater than or equal to 20% bone marrow or peripheral blood blasts cells that may have morphological features of most AML, NOS categories and has a higher frequency among cases with minimal differentiation.

Definitive Diagnostic Methods

Cytogenetics
Genetic testing

Genetics Data

ASXL1
FLT3-ITD
IDH1R132
Karyotypic abnormalities, most commonly trisomies 8 and 13
KMT2A
Mutated RUNX1

Immunophenotyping

Immunophenotyping not relevant, diagnosed via genetic analysis

Treatments

Hematologic Transplant and/or Endocrine Procedures

Transformations to

None

Transformations from

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C92.0 Acute myeloid leukemia

Corresponding ICD-10-CM Codes (U.S. only)

C92.0 Acute myeloblastic leukemia (effective October 01, 2015 - September 30, 2024)
C92.00 Acute myeloblastic leukemia not having achieved remission (effective October 01, 2024)
C92.01 Acute myeloblastic leukemia, in remission (effective October 01, 2024)
C92.02 Acute myeloblastic leukemia, in relapse (effective October 01, 2024)

Signs and Symptoms

Anemias
Bone marrow failure
Easy bruising or bleeding
Fatigue
Fever
Leukocytosis
Neutropenia
Petechiae
Shortness of breath
Thrombocytopenia
Weakness
Weight loss or loss of appetite

Diagnostic Exams

Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Immunophenotyping
Lumbar puncture
Peripheral blood smear
Physical exam and history
Reverse transcription-polymerase chain reaction test (RT-PCR)

Progression and Transformation

None

Epidemiology and Mortality

Age: Higher frequence in older adults (>60 years)
Incidence: 4-16% of AML cases
Survival: Worse overall survival

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Acute myeloid leukemia and related precursor neoplasms
Pages: 144-145

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Acute Myeloid Leukemia Treatment. Bethesda, MD: National Cancer Institute. Updated <03/06/2024>. Available at: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq. Accessed <02/06/2025>. [PMID: 26389432]
Section: Acute Myeloid Leukemia Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/leukemia/hp/adult-aml-treatment-pdq
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